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Reflex myoclonic epilepsy in infancy: a benign age-dependent idiopathic startle epilepsy

Dimitrios Zafeiriou1, Euthymia Vargiami, Eleftherios Kontopoulos

  • 11st Pediatric Clinic, Aristotle University of Thessaloniki, Greece. jeff@med.auth.gr

Epileptic Disorders : International Epilepsy Journal with Videotape
|July 24, 2003
PubMed

Insights

Reflex myoclonic epilepsy of infancy (RMEI) is a rare condition triggered by stimuli. Early treatment with sodium valproate led to complete remission in a young patient.

Area of Science:

  • Neurology
  • Pediatric Neurology
  • Epileptology

Background:

  • Reflex myoclonic epilepsy of infancy (RMEI) is an idiopathic epileptic disorder.
  • Characterized by myoclonic attacks in the first two years of life.
  • Attacks are precipitated by unexpected tactile or auditory stimuli.

Observation:

  • A 9-month-old infant presented with reflex myoclonic attacks.
  • Attacks occurred in clusters of symmetric jerks, primarily affecting arms and head.
  • Stimuli included unexpected auditory triggers; no family history of epilepsy.
  • Ictal EEG showed a 3 Hz spike-wave pattern; EEG during wakefulness/sleep was normal.
  • Neurodevelopmental examination and brain MRI were unremarkable.

Findings:

  • The infant's myoclonic attacks resolved within 3 weeks of initiating sodium valproate.
  • No recurrence of seizures observed during a 3-year, 3-month follow-up.
  • The patient exhibited normal psycho-motor development at 4 years of age.

Implications:

  • RMEI is an age-dependent, idiopathic, generalized epileptic syndrome.
  • The condition appears to have a favorable prognosis with appropriate treatment.
  • Highlights the efficacy of sodium valproate in managing RMEI.

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